What is Adams Oliver Syndrome?

Adams Oliver Syndrome is a disorder characterized by scalp and skull defects and abnormalities of the limbs, fingers, and/or toes. The physical abnormalities vary greatly among children. Some cases may be very mild while others are severe. Babies with Adams Oliver Syndrome have scalp defects that are present at birth. These defects can include multiple bare, exposed areas that have dilated blood vessels under the skin. Underlying bony defects in the skull may also be present in the most severe cases. The limb defects can include abnormally short (hypoplastic) limbs, absent hands, and absent lower legs. Defects of the fingers and toes can include short bones, missing digits, widely spaced digits, and clubfoot.

How Many Children Have Adams Oliver Syndrome?

It is an extremely rare condition affecting boys and girls equally.

How Do You Know If Your Child Has Adams Oliver Syndrome?

At this time, no specific pre-natal tests are available for diagnosing this condition. An ultrasound scan may detect the more severe cranial and limb defects. Physical abnormalities will be evident at birth.

What Causes Adams Oliver Syndrome?

Most cases are inherited as an autosomal dominant trait (one parent carries a dominant gene), but there are also reports of autosomal recessive (both parents must carry a recessive gene) inheritance. Some cases occur sporadically as the result of a spontaneous genetic change. Although the underlying cause of the condition is not known, it has been suggested that it could result from the interruption of early embryonic blood supply in the arteries under the collar bone. Genetic counseling is recommended for families affected by this condition.

How Can You Help a Child with Adams Oliver Syndrome?

Treatment for your child usually focuses on improving the limb and skull defects. Surgery and/or prostheses (artificial limbs) might be necessary for limb defects and scalp surgery might be necessary for skull defects. Use of a helmet during physical activities is often beneficial for protecting your child’s scalp.

What’s in the Future for a Child with Adams Oliver Syndrome?

Scalp and skull defects present at birth often heal spontaneously in the first few months of life, but in more serious cases plastic surgery might be required. Although intellectual development is usually normal, some cases of mental retardation and learning disabilities have been reported. A normal life span is expected for most children with Adams Oliver Syndrome.

Fact Sheet by:

Birth Defect Research Children, Inc.
www.birthdefects.org